We report the case of a 12 y.o. male patient with 49XXXXY syndrome, previously unknown as an epileptic, but presented irritability and aggressive behaviour and was suspected of obstructive sleep apneas. Polygraphic recording ruled out apneas and showed bilateral independent interictal epileptiform abnormalities. During intermittent photic stimulation a generalized tonic-clonic seizure was elicited. MRI showed multiple hypointense white matter areas. CBZ improved cognitive performances and EEG.
